Rajendran C P, Porkodi R, Parthiban R
Department of Rheumatology, Madras Medical College, Chennai.
J Assoc Physicians India. 2001 Apr;49:412-4.
To study the clinical and immunological profile of mixed connective tissue disease (MCTD) in rheumatic disease population.
We retrospectively analyzed 6400 cases of rheumatic disease population who took treatment in the Department of Rheumatology, Madras Medical College, Chennai during the period of 1996 to 1999, in which eight cases fulfilled the preliminary diagnostic criteria of mixed connective tissue disease devised by Kasukawa et al. All eight cases were studied in detail.
All cases were females between 23 to 50 years of age. Polyarthritis, Raynaud's phenomenon and sclerodactyly were present in all eight patients. Oesophageal abnormalities, pulmonary changes and myositis were present in six patients. Facial erythema was observed in five patients. Alopecia and oral ulcers were seen in four patients. Two patients had pulmonary hypertension and migraine like headache. One patient had diffuse proliferative glomerulonephritis as an interesting feature by renal biopsy. Pleuritis, pericarditis and trigeminal neuropathy with lower cranial nerve palsies were present in one case each. Immunological tests showed presence of antinuclear antibodies and anti U1 ribonucleoprotein (anti U1RNP) antibodies in all eight patients.
Mixed connective tissue disease should be considered as an important syndrome in any patient who presents with heterogeneous clinical presentation and who do not fit into any definite criteria of systemic connective tissue disorders.
研究风湿性疾病人群中混合性结缔组织病(MCTD)的临床和免疫学特征。
我们回顾性分析了1996年至1999年期间在金奈马德拉斯医学院风湿病科接受治疗的6400例风湿性疾病患者,其中8例符合Kasukawa等人制定的混合性结缔组织病初步诊断标准。对所有8例患者进行了详细研究。
所有病例均为23至50岁的女性。所有8例患者均出现多关节炎、雷诺现象和指(趾)硬皮病。6例患者出现食管异常、肺部改变和肌炎。5例患者观察到面部红斑。4例患者出现脱发和口腔溃疡。2例患者有肺动脉高压和偏头痛样头痛。1例患者经肾活检发现弥漫性增殖性肾小球肾炎这一有趣特征。胸膜炎、心包炎和伴有下颅神经麻痹的三叉神经病变各有1例。免疫学检查显示所有8例患者均存在抗核抗体和抗U1核糖核蛋白(抗U1RNP)抗体。
对于临床表现多样且不符合任何系统性结缔组织疾病明确标准的患者,应将混合性结缔组织病视为一种重要的综合征。